Ishigami Keisuke, Shitani Masahiro, Kimura Yasutoshi, Hasegawa Tadashi, Masaki Yoshiharu, Ito Ayako, Akutsu Noriyuki, Yamamoto Motohisa, Motoya Masayo, Sasaki Shigeru, Takahashi Hiroki, Takemasa Ichiro, Nakase Hiroshi
Department of Gastroenterology and Hepatology.
Department of Surgery, Surgical Oncology and Science.
Medicine (Baltimore). 2018 Dec;97(52):e13868. doi: 10.1097/MD.0000000000013868.
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a chronic inflammatory disorder characterized by high levels of serum IgG4, swollen organs with fibrosis and abundant infiltration of IgG4-positive plasmacytes.
An 82-year-old male visited our hospital for an evaluation of a pancreatic enlargement and a bilateral submandibular adenopathy. Further investigation revealed elevation of serum IgG4 and bilateral lacrimal submandibular adenopathy. We diagnosed him with IgG4-related disease (IgG4-RD) and started administration of corticosteroid (CS) therapy. Both pancreatic enlargement and adenopathy rapidly improved; however, there was a new occurrence of diffuse wall thickening of the gallbladder during CS treatment.
Radiological examination revealed diffuse wall thickening of the gallbladder, and its inner layer was smooth and homogenous. These findings suggested an inflammatory change, but the possibility of malignancy could not be excluded.
The patient underwent laparoscopic cholecystectomy for a pathological diagnosis.
Histological examination revealed a transmural infiltration of IgG4 positive plasma cells and dense fibrosis. The patient was pathologically diagnosed with IgG4 related cholecystitis presenting as an ectopic relapse.
There are 2 major types of IgG4-related cholecystitis, a diffuse wall thickening type and a mass formation type. It is sometimes difficult to differentiate IgG4-related cholecystitis with gallbladder cancer.Corticosteroid (CS) is effective for induction of remission; however, we sometimes encounter disease relapse after reduction of CS dose. We should be mindful that some patients may relapse with new organ involvements even if the primary site and serum IgG4 level are well controlled.
免疫球蛋白（Ig）G4相关疾病（IgG4-RD）是一种慢性炎症性疾病，其特征为血清IgG4水平升高、器官肿大伴纤维化以及IgG4阳性浆细胞大量浸润。
一名82岁男性因胰腺肿大和双侧颌下腺病变来我院就诊。进一步检查发现血清IgG4升高以及双侧泪腺和颌下腺病变。我们诊断他患有IgG4相关疾病（IgG4-RD）并开始给予糖皮质激素（CS）治疗。胰腺肿大和腺病迅速改善；然而，在CS治疗期间胆囊出现了新的弥漫性壁增厚。
影像学检查显示胆囊弥漫性壁增厚，其内层光滑且均匀。这些发现提示有炎症改变，但不能排除恶性肿瘤的可能性。
患者接受了腹腔镜胆囊切除术以进行病理诊断。
组织学检查显示IgG4阳性浆细胞全层浸润和致密纤维化。患者经病理诊断为表现为异位复发的IgG4相关胆囊炎。
IgG4相关胆囊炎主要有两种类型，弥漫性壁增厚型和肿块形成型。有时难以将IgG4相关胆囊炎与胆囊癌区分开来。糖皮质激素（CS）对诱导缓解有效；然而，我们有时会在CS剂量减少后遇到疾病复发。我们应该注意，即使原发部位和血清IgG4水平得到良好控制，一些患者仍可能因新的器官受累而复发。
